German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease: Report 2021 and 9 Years' Longitudinal Observations on Fallot and Coarctation Patients.

BACKGROUND: The annual report of the German Quality Assurance of Congenital Heart Disease displays a broad overview on outcome of interventional and surgical treatment with respect to patient's age and risk categorization. Particular features of the German all-comers registry are the inclusion of all interventional and surgical procedures, the possibility to record repeated treatments with distinct individual patient assignment, and to record various procedures within one case. METHODS: International Pediatric and Congenital Cardiac Code terminology for diagnoses and procedures as well as classified adverse events, also recording of demographic data, key procedural performance indicators, and key quality indicators (mortality, adverse event rates). Surgical and interventional adverse events were classified according to the Society of Thoracic Surgeons and to the Congenital Heart Disease Adjustment for Risk Method of the congenital cardiac catheterization project on outcomes. Annual analysis of all cases and additional long-term evaluation of patients after repair of Fallot and primary treatment of native coarctation of the aorta were performed. RESULTS: In 2020, 5,532 patients with 6,051 cases (hospital stays) with 6,986 procedures were treated in 23 German institutions. Cases dispense on 618 newborns (10.2%), 1,532 infants (25.3%), 3,077 children (50.9%), and 824 adults (13.6%). Freedom from adverse events was 94.5% in 2,795 interventional cases, 67.9% in 2,887 surgical cases, and 42.9% in 336 cases with multiple procedures (without considering the 33 hybrid interventions). In-hospital mortality was 0.5% in interventional, 1.6% in surgical, and 5.7% in cases with multiple treatments. Long-term observation of 1,632 patient after repair of Fallot depicts the impact of previous palliation in 18% of the patients on the rate of 20.8% redo cases. Differentiated analysis of 1,864 patients with native coarctation picture clear differences of patient, age, and procedure selection and outcome. The overall redo procedure rate in this patient population is high with 30.8%. CONCLUSION: Improvement in quality of care requires detailed analysis of risks, performance indicators, and outcomes. The high necessity of redo procedures in patients with complex congenital heart disease underlines the imperative need of long-term observations.

Aortic stenosis of the neonate: A single-center experience.

OBJECTIVES: Because data for neonates are limited, optimal management of critical aortic stenosis remains controversial (balloon valvotomy [BV] or open valvoplasty [OV]). In a center with balanced experience in both methods, we hypothesized that OV can provide a better individualized approach than blunt BV and better serve long-term outcomes. METHODS: A retrospective review of data and follow-up (survival, freedom from operation/replacement) of all neonates, suitable for biventricular repair, undergoing aortic valve procedure (1989-2015), was performed. RESULTS: One hundred three patients were concomitantly treated (BV [n = 51], OV [n = 52). Median age was 8 days, median aortic annulus Z-score was -1.3 for BV (range, -3.9 to 2.0) and OV (-3.9 to 3.2) groups. Operative mortality after BV or OV was 8% (n = 4) and 4% (n = 2), respectively. With a 13-year median follow-up, 10-year freedom from operation was 36% and 66% after BV or OV, respectively. Valve replacement was ultimately required in 32 patients (n = 20 [39%] in the BV group; n = 12 [23%] in the OV group) within a 5.9-year median time. After OV, tricuspid arrangement of the repaired aortic valve provided a 10-year freedom from operation and replacement of 87% and 95%, respectively. In multivariate analysis, associated left heart malformations, BV, nontricuspid geometry, and inadequate post procedural result were predictive of operation and replacement. CONCLUSIONS: In neonates with critical aortic stenosis, both methods (BV and OV) offer excellent survival benefit. OV significantly minimizes the need for operation, whereas BV did not postpone age of replacement. Clearly superior results are achieved with OV when a post repair tricuspid arrangement is obtained.

Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy.

OBJECTIVES: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. METHODS: From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% <1 year); 86 (45%) had a preceding balloon valvuloplasty. The indications for the procedure were stenotic (n = 123; 64%), regurgitant (n = 63; 33%) or combined (n = 7; 4%) disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post-repair geometry was tricuspid in 137 (71%) patients. RESULTS: The 10-year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47-66) and 68% (95% confidence interval, 59-76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non-tricuspid post-repair geometry and cross-clamp duration were predictors for reoperation and replacement. After a mean follow-up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%). CONCLUSIONS: Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes.

Current mid-term outcome with an integrated surgical strategy for correction of d-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: The optimal surgical treatment strategy for transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) leading to the best long-term outcome has not been established yet. The wide range of anatomical variability has led to the development of different surgical treatment options. In a retrospective single-centre review, we aimed to report the long-term outcome in patients who underwent biventricular repair. METHODS: Between 1992 and 2015, 49 patients underwent biventricular repair for TGA with VSD and LVOTO. On the basis of anatomical substrate, the type of repair was chosen. According to the type of repair, the patients were divided into two groups. Group A (n = 30 patients) includes all patients who underwent intraventricular rerouting (Rastelli, n = 22; réparation à l'étage ventriculaire, n = 8); Group B patients (n = 14) had repair on an arterial level (arterial switch operation + VSD closure + LVOTO resection, n = 10; Bex-Nikaidoh, n = 4). RESULTS: The follow-up was completed in 90% with a median follow-up of 12.3 years (17.1-17.7 years). There was no difference in survival (Group A: 97% at 5, 10 and 15 years; Group B: 92% at 5, 10 and 15 years) and functional outcome between the groups. All patients were in New York Heart Association class I, having sinus rhythm and normal left ventricular (LV) function. Neurological and educational development was adequate for age in all but one who suffered from attention-deficit hyperactivity disorder. Group A exhibited significantly less freedom from right ventricular outflow tract (RVOT) reoperations/reintervention (79, 48 and 21% at 5, 10 and 15 years) (P = 0.008) due to RVOT dysfunction and endocarditis. Event-free LVOTO survival in Group A at 5, 10 and 15 years amounted to 96, 91 and 91% and was similar to Group B with event-free LVOTO survival at 5, 10 and 15 years of 100, 88 and 88%, respectively. CONCLUSIONS: LV function is well preserved irrespective of the type of procedure. Patients who had undergone intraventricular rerouting (Group A) suffered from recurrent RVOT interventions due to RVOT dysfunction and endocarditis. The neurological outcome and the educational level were adequate for age in both groups. Executing the appropriate type of surgery for the individual patient's anatomy provides excellent functional and event-free outcome.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia.

BACKGROUND: Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. PATIENTS AND METHODS: A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. RESULTS: Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). CONCLUSION: Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Reimbursement by current German diagnosis-related groups system penalises complex congenital heart surgery.

A total of 458 hospital stays during the year 2011 were analysed to determine whether reimbursement by the current German Diagnosis-Related Groups system covers the costs incurred during hospital stay for congenital heart surgery. The costs of every hospital stay were estimated according to the guidelines of the Institute for the Hospital Remuneration System, an institute responsible for encoding hospital reimbursement in Germany. Cost-weight values of the year 2012 were applied for reimbursement. Related additional compensations were also included. Hospital costs ranged from 8896.26 to 193,671.94 euros per case, with a mean of 30,597 and standard deviation of 25,032 euros. Reimbursement varied from 8630.35 to 173,710.65 euros, with a mean of 25,514 and standard deviation of 18,497 euros: an underfunding of 17%. Fifty-nine per cent (271/458) of cases were classified, according to Aristotle complexity score, in higher comprehensive complexity: Levels 4-6. Costs highly correlated with complexity levels (Spearman's r coefficient = 0.89) and the regression was linear. Underfunding increased, linearly, from 6% for procedures with Level 1, lowest comprehensive complexity, to 23% for those with Level 6, highest complexity. In conclusion, this study demonstrates that reimbursement by the current German Diagnosis-Related Groups system increasingly penalises complex congenital heart surgery. Aristotle complexity score could help to correct this prejudicial situation.

Is there any need for a shunt in the treatment of tetralogy of Fallot with one source of pulmonary blood flow?

OBJECTIVES: In symptomatic patients, performing a primary repair of tetralogy of Fallot (TOF), irrespective of age or placing a shunt, remains controversial. The aim of the study was to analyse the policy of primary correction. METHODS: Between May 2005 and May 2012, a total of 87 consecutive patients with TOF, younger than 6 months of age, underwent primary correction. All patients had one source of pulmonary blood flow, with or without a patent ductus arteriosus. The median age at surgery was 106 ± 52.3 days (8-180 days). Twelve patients (13.8%) were newborns. Two groups were analysed: group I, patients <1 month of age; group II, patients between 2-6 months of age. RESULTS: There was no early or late death at 7 years of follow-up. There was no difference in bypass time or hospital stay between the two groups, but the Aristotle comprehensive score (P < 0.0001), ICU stay (P = 0.030) and the length of ventilation (P = 0.014) were significantly different. Freedom from reoperation was 87.3 ± 4.3% and freedom from reintervention was 85.9 ± 4.2% at 7 years, with no difference between the two groups. Neurological development was normal in all patients, but 1 patient in Group II had cerebral seizures and showed developmental delay. Growth was adequate in all patients, except those with additional severe non-cardiac malformations that caused developmental delay. Eighty-five per cent of the patients were without cardiac medication. CONCLUSIONS: Even in symptomatic neonates and infants <6 months of age, primary repair of TOF can be performed safely and effectively. One hundred per cent survival at 7 years suggests that early primary repair causes no increase in mortality in the modern era. Shunting is not necessary, even in symptomatic newborns, thus avoiding the risk of shunt-related complications and repeated hospital stays associated with a staged approach.

Quantification of morbidity associated with congenital heart surgery.

OBJECTIVE: The objective of this study was to estimate the morbidity according to observed complications after congenital heart surgery over 1-year period. METHODS: The previously established list of conditions prone to affect patients' well-being or increase cost of in-hospital stays was used systematically to score the severity of postoperative complications from 1 to 4 points. The morbidity score was calculated by adding the scores of observed complications. When the sum amounted to more than 5 points, a morbidity score of only 5 points was attributed. If no complication was detected, a score of 0.5 points was assigned. The resulting morbidity scores were correlated with the length of stay in the intensive care unit (ICU) and in the hospital, the duration of mechanical ventilation, and Aristotle complexity scores. RESULTS: A total of 542 primary procedures performed in the year 2011 were studied. Aristotle basic and comprehensive scores amounted to 7.78 ± 2.65 and 10.15 ± 3.83, respectively. Mortality was 1.85% (10/542). The standardized ratio of surgical performance reached 103.10%. Total cavopulmonary connection with extracardiac fenestrated conduit constituted the most frequent operation (n = 34). No complication was observed following 183 (33.8%) procedures. More than two complications were observed in 114 cases (21%). The three most frequent unfavorable conditions were "mechanical ventilation 25 to 95 hours" (n = 150), low cardiac output syndrome (n = 56), and cardiac arrhythmia requiring medication (n = 50). The estimated mean morbidity score amounted to 2.26 ± 1.80 points. Scores ranged from 0.68 ± 0.50 for primary closure of atrial septal defect to 4.50 ± 0.79 for the Norwood procedure. They were perfectly related to the length of ICU stay and to the duration of mechanical ventilation (Spearman coefficient r = 1). Correlation was high with the length of hospital stay (r = 0.83), Aristotle basic score (r = 0.89) (r = 0.96), and comprehensive score (r = 0.94) (C-index = 0.97). The observed mean morbidity score was statistically not different from the expected mean morbidity score according to the basic Aristotle complexity: p = 0.73. CONCLUSION: Quantification of morbidity indicates the length of ICU stay and the duration of mechanical ventilation as the best surrogates for morbidity. Such benchmarking and scoring of observed postoperative complications paves the way for an accurate assessment and improvement of quality care in congenital heart surgery.

The long-term outcome of open valvotomy for critical aortic stenosis in neonates.

BACKGROUND: This study analyzed the long-term outcome after operations for open aortic valvotomy, specifically looking at the preservation of the native aortic valve. METHODS: Between 1983 and 2011, 34 neonates with critical aortic stenosis underwent open valvotomy. The mean age at operation was 15 days (range, 1 to 28 days). Follow-up was 100% complete. RESULTS: Three patients died early. The probability of survival was 91.2%, with a mean follow-up of 11 years (range, 10 months to 21 years). Event-free survival was 77%, 68%, 68%, and 57% at 5, 10, 15, and 20 years, respectively, and freedom from aortic valve replacement was 93%, 83%, 68%, and 57%. Tricuspid valve morphology revealed the best outcome, with event-free survival of 90% (p = 0.006) and 100% freedom from aortic valve replacement (p = 0.0012) at 20 years of follow-up. All but 4 patients were in New York Heart Association class I; 77 % of all patients were without medication. CONCLUSIONS: In neonates, predictable and consistent long-term results can be accomplished by open valvotomy in any type of valve morphology, even in an asymmetric arrangement. Operative repair not only offers a 90% survival benefit in the long-term but also preserves the native aortic valve in most patients. Clearly superior results were achieved in tricuspid valve morphology, in which there was no need for aortic valve replacement at 20 years of follow-up.

A morbidity score for congenital heart surgery based on observed complications.

OBJECTIVES: The aim of this study was to develop a morbidity score based on observed postoperative complications after congenital heart surgery. METHODS: Conditions or diseases that impair patients after congenital heart surgery or increase costs of hospital stay were called complications and attributed to scores ranging from 1 (mild) to 4 (severe) points, according to estimated severity or costliness. 'No complication' was assigned 0.5 points. From January to March 2011, scores for each observed 'complication' for every main (primary) surgical procedure were recorded and a morbidity score was calculated. In conformity with the Aristotle score methodology, if the sum of observed complication scores amounted to more than 5 points, a morbidity score of only 5 points was attributed. The estimated morbidity score was compared with the morbidity score attributed by the Aristotle basic complexity (ABC) score. RESULTS: One hundred and thirty-nine primary procedures were carried out. The mean ABC and Aristotle comprehensive complexity scores reached 8.31 ± 2.52 and 9.62 ± 3.47 points, respectively. Two patients died. No complication was detected after 46 procedures. Overall, there were 185 listed 'morbidity' conditions in connection with the other 93 surgical procedures, rendering a total score of 385 points. The most frequent event was 'mechanical ventilation 25-95 h': n = 39. The mean morbidity score was 2.14 ± 1.63. The morbidity scores ranged from 0.5 points (n = 46) to 5 points (n = 23) with a median of 2.0 points. The scores for 11 different procedures that were performed at least five times positively correlated with the corresponding Aristotle morbidity scores: Pearson's coefficient r = 0.75. But the morbidity score for bidirectional cavopulmonary anastomosis (3.14 ± 1.77) was higher than the corresponding Aristotle morbidity score (2.0). It was lower for 'conduit placement, right ventricle to pulmonary artery': 1.08 ± 0.97, versus 2.0, and for arterial switch operation: 2.08 ± 1.11, versus 3.0. CONCLUSIONS: The reported morbidity scores need to be tested on larger series and in different institutions. The introduced morbidity score has the potential to quantify postoperative complications accurately. Its estimation over time can facilitate the assessment of quality of congenital heart surgery. It will allow comparison of morbidity outcomes across institutions with different case-mixes.

Surgical management of congenital heart disease: contribution of the Aristotle complexity score to planning and budgeting in the German diagnosis-related groups system.

Planning and budgeting for congenital heart surgery depend primarily on how closely reimbursement matches costs and on the number and complexity of the surgical procedures. Aristotle complexity scores for the year 2010 were correlated with hospital costs and with reimbursement according to the German diagnosis-related groups (DRG) system. Unit surgical performance was estimated as surgical performance (complexity score × hospital survival) times the number of primary procedures. This study investigated how this performance evolved during years 2006 to 2010. Hospital costs and reimbursements correlated highly with Aristotle comprehensive complexity levels (Spearman r = 1). Mean costs and reimbursement reached 35,050 ± 32,665 and 31,283 ± 34,732, respectively, for an underfunding of 10.7%. Basic and comprehensive unit surgical performances were respectively 3036 ± 1009 and 3891 ± 1591 points in 2006. Both performances increased in sigmoid fashion to reach 3883 ± 1344 and 5335 ± 1314 points, respectively, in 2010. Top performances would be achieved in year 2011, and extrapolated costs would comprise about 19,434,094.92 (95% confidence interval, 11,961,491.22-22,495,764.42). The current underfunding of congenital heart surgery needs correction. The Aristotle score can help to adjust reimbursement according to complexity of procedures. Unit surgical performance allows accurate budgeting in the current German DRG system.

Congenital heart surgery: surgical performance according to the Aristotle complexity score.

OBJECTIVES: Aristotle score methodology defines surgical performance as 'complexity score times hospital survival'. We analysed how this performance evolved over time and in correlation with case volume. METHODS: Aristotle basic and comprehensive complexity scores and corresponding basic and comprehensive surgical performances were determined for primary (main) procedures carried out from 2006 to 2009. Surgical case volume performance described as unit performance was estimated as 'surgical performance times the number of primary procedures'. RESULTS: Basic and comprehensive complexity scores for the whole cohort of procedures (n=1828) were 7.74±2.66 and 9.89±3.91, respectively. With an early survival of 97.5% (1783/1828), mean basic and comprehensive surgical performances reached 7.54±2.54 and 9.64±3.81, respectively. Basic surgical performance varied little over the years: 7.46±2.48 in 2006, 7.43±2.58 in 2007, 7.50±2.76 in 2008 and 7.79±2.54 in 2009. Comprehensive surgical performance decreased from 9.56±3.91 (2006) to 9.22±3.94 (2007), and then to 9.13±3.77 (2008), thereafter increasing up to 10.62±3.67 (2009). No significant change of performance was observed for low comprehensive complexity levels 1-3. Variation concerned level 4 (p=0.048) which involved the majority of procedures (746, or 41% of cases) and level 6 (p<0.0001) which included a few cases (20, or 1%), whereas for level 5, statistical significance was almost attained: p=0.079. With a mean annual number of procedures of 457, mean basic and comprehensive unit performance was estimated at 3447±362 and 4405±577, respectively. Basic unit performance increased year to year from 3036 (2006, 100%) to 3254 (2007, 107.2%), then 3720 (2008, 122.5%), up to 3793 (2009, 124.9%). Comprehensive unit performance also increased: from 3891 (2006, 100%) to 4038 (2007, 103.8%), 4528 (2008, 116.4%) and 5172 (2009, 132.9%). CONCLUSIONS: Aristotle scoring of surgical performance allows quality assessment of surgical management of congenital heart disease over time. The newly defined unit performance appears to well reflect the trend of activity and efficiency of a congenital heart surgery department.

The modified Senning procedure as an integral part of an anatomical correction of congenitally corrected transposition of the great arteries.

In the current era of anatomical correction of complete transposition of the great arteries, the Senning operation is reserved only for the atrial switch part of anatomical corrections of congenitally corrected transposition of the great arteries. These rare operations are performed in only a few centers all over the world; the majority of practicing cardiac surgeons therefore have limited experience with the Senning operation. The proposed modified Senning procedure might simplify the original concept. Once the technical aspect of the procedure is accomplished, the risk of systemic and pulmonary baffle obstructions is minimal, even in situs solitus with dextrocardia or situs inversus with levocardia. Furthermore, this technique has the potential to provide adequate capacity of the pulmonary venous atrium, to preserve optimal geometry of the mitral valve, to minimize damage of sinus node and to make the coronary sinus accessible for electrophysiological studies or intervention by keeping the coronary sinus in the systemic venous baffle. The modified technique is simple, highly reproducible and applicable, regardless of the situs and position of the apex of the heart.

Congenital heart disease: interrelation between German diagnosis-related groups system and Aristotle complexity score.

OBJECTIVES: The Disease-Related Groups (DRGs) system postulates that inpatient stays with similar levels of clinical complexity are expected to consume similar amounts of resources. This, applied to surgery of congenital heart disease, suggests that the higher the complexity of procedures as estimated by the Aristotle complexity score, the higher hospital reimbursement should be. This study analyses how much case-mix index (CMI) generated by German DRG 2009 version correlates with Aristotle score. METHODS: A total of 456 DRG cases of year 2008 were regrouped according to German DRG 2009 and related cost-weight values and overall CMI evaluated. Corresponding Aristotle basic and comprehensive complexity scores (ABC and ACC) and levels were determined. Associated surgical performance (Aristotle score times hospital survival) was estimated. Spearman 'r' correlation coefficients were calculated between Aristotle scores and cost-weights. Goodness of fit 'r(2)' from derived regression was determined. Correlation was estimated to be optimal if Spearman 'r' and derived goodness of fit 'r(2)' approached 1 value. RESULTS: CMI was 8.787 while mean ABC and ACC scores were 7.64 and 9.27, respectively. Hospital survival was 98.5%: therefore, surgical performance attained 7.53 (ABC score) and 9.13 (ACC score). ABC and ACC scores and levels positively correlated with cost-weights. With Spearman 'r' of 1 and goodness of fit 'r(2)' of 0.9790, scores of the six ACC levels correlated at best. The equation was y = 0.5591 + 0.939x, in which y stands for cost-weight (CMI) and x for score of ACC level. CONCLUSIONS: ACC score correlates almost perfectly with corresponding cost-weights (CMI) generated by the German DRG 2009. It could therefore be used as the basis for hospital reimbursement to compensate in conformity with procedures' complexity. Extrapolated CMI in this series would be 9.264. Modulation of reimbursement according to surgical performance could be established and thus 'reward' quality in congenital heart surgery.

Surgery for tetralogy of Fallot - absent pulmonary valve syndrome. Technique of anterior translocation of the pulmonary artery.

Patients with absent pulmonary valve syndrome (APVS) and tetralogy of Fallot represent a particular management's challenge. The distinctive feature of APVS is the airway obstruction caused by tracheobronchial compression that results from massive dilatation of the main pulmonary artery (PA) and its first- and second-order branches. Consequential tracheomalacia and bronchomalacia determine the timing and severity of respiratory compromise, as well as the morbidity and mortality of these patients. Although several techniques have been proposed for decompression of the airways, the method of choice is still controversial. Translocation of the PA anterior to the aorta and away from the airways has the potential to reduce or eliminate bronchial compression. Overall, the intermediate-term functional outcomes using this technique have been encouraging, with zero mortality even in the youngest age group of patients and the disappearance of respiratory symptoms in the majority of them. Nevertheless, failure of the treatment could be expected in symptomatic patients in whom the pathology of the airways extends beyond the proximal PAs.

Surgical management of congenital heart disease: evaluation according to the Aristotle score.

OBJECTIVE: The Aristotle basic complexity (ABC) score (1.5-15 points) is the sum of potentials for early mortality, morbidity and anticipated surgical technique difficulty. The Aristotle comprehensive complexity (ACC) score (1.5-25 points) is the sum of ABC score and patient-adjusted complexity score; it comprises six complexity levels. We used the ACC score to evaluate quality in surgical management of congenital heart disease. METHODS: Procedures performed in year 2002 and 2007 were analysed. Proportion of procedures requiring at least 1 week of stay in the intensive care unit was chosen as the marker of morbidity. We adopted threshold duration of 120 min for cardio-pulmonary bypass (CPB) cases and the same duration for operations without CPB as surrogate of surgical technical difficulty. The ACC scores were correlated to mortality, morbidity and technical difficulty. RESULTS: This study included 758 patients who underwent 787 primary procedures. The mean ABC and ACC scores amounted to 7.61+/-2.46 and 9.51+/-3.84. Early mortality was 3.05% (24/787), 95% confidence interval (CI): 1.97-4.51%. Zero at ACC levels 1 and 2, it increased from 1.2% (2/161) for level 3 up to 22.2% (2/9) for level 6. Morbidity index was evaluated at 25.9% (204/787), 95% CI: 22.9-29.1%. 1.9% at level 1, it escalated up to 77.8% at level 6. Index of technique difficulty was estimated at 35.2% (277/787), 95% CI: 31.8-38.6%, ranging from 4.8% for level 1 to 66.7% for level 6. A high correlation was found between the ACC scores and mortality, indices of morbidity and technique difficulty, Spearman's correlation coefficient r being 0.9856, 1 and 0.9429, respectively. Mortality (p=0.037) and morbidity (p=0.041) were lower in year 2007 than in 2002 with ABC (p=0.18) and ACC (p=0.37) surgical performance being not significantly different. CONCLUSIONS: The Aristotle score is still under development. Morbidity evaluation should be ideally based on observed postoperative complications; estimation of surgical technical difficulty chosen in this study may not be generalised. Nevertheless, the actual Aristotle comprehensive complexity score, as evaluated in its three components, accurately determined the outcome of surgical management of congenital heart disease. It appears to be an adequate tool to evaluate quality in paediatric cardiac surgery, over time.

Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle.

Totally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.